Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Angiomyolipoma, Mediastinal

  • Ronda Sanders
  • Merce JordaEmail author
Reference work entry

Angiomyolipoma is a usually benign mesenchymal neoplasm composed of smooth muscle, blood vessels, and adipose tissue in varying amounts (Morita et al. 2012). It occurs predominantly in the kidney but can rarely occur in extrarenal locations including the liver, lungs, and the mediastinum (Candaș et al. 2013). Though they may be sporadic in origin, angiomyolipomas are associated with tuberous sclerosis and TSC2/PKD1 contiguous gene syndrome (Martignoni et al. 2002). Patients with a genetic syndrome tend to present at a younger age than patients without a genetic mutation. Angiomyolipomas belong to the perivascular epithelioid cell tumor (PEComa) family, which means they have perivascular and epithelioid features and can coexpress melanocytic and muscle markers. The PEComa family includes angiomyolipoma (AML), clear-cell sugar tumor (CCST) of the lung, lymphangioleiomyomatosis (LAM), clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and other rare clear-cell...

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References and Further Reading

  1. Candaș, F., Berber, U., Yildizhan, A., Yiyit, N., Görür, R., & Ișitmangil, T. (2013). Anterior mediastinal angiomyolipoma. The Annals of Thoracic Surgery, 95(4), 1431–1432.CrossRefPubMedGoogle Scholar
  2. Liang, W., Xu, S., & Chen, F. (2015). Malignant perivascular epithelioid cell neoplasm of the lung: One case report. Medicine (Baltimore), 94(22), 904.CrossRefGoogle Scholar
  3. Llarena Ibarguren, R., et al. (1991). Renal angiomyolipoma with lymphatic involvement. Archivos Españoles de Urología, 44(1), 75–76.PubMedGoogle Scholar
  4. Martignoni, G., et al. (2002). Renal disease in adults with TSC2/PDK1 contiguous gene syndrome. The American Journal of Surgical Pathology, 26(2), 198–205.CrossRefPubMedGoogle Scholar
  5. Martignoni, G., Pea, M., Reghellin, D., et al. (2008). PEComas: The past, the present and the future. Virchows Archiv, 452(2), 119–132.CrossRefPubMedGoogle Scholar
  6. Morita, K., et al. (2012). Angiomyolipomas of the mediastinum and lung. Journal of Thoracic Imaging, 27(1), W21–W23.CrossRefPubMedGoogle Scholar
  7. Park, J. H., Lee, C., Suh, J. H., Kim, G., Song, B., & Moon, K. C. (2016). Renal epitheliod angiomyolipoma: Histopathologic review, immunohistochemical evaluation and prognostic significance. Pathology International, 66(10), 571–577.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory MedicineUniversity of Miami Miller School of MedicineMiamiUSA
  2. 2.Department of UrologyUniversity of Miami Miller School of MedicineMiamiUSA